It is well known that pulmonary pathophysiology is one of the most significant factors associated with the morbidity and mortality of individuals afflicted with either acute or chronic spinal cord injuries (SCI). Furthermore, abnormalities of respiratory function and failure of the respiratory system are leading causes of mortality in the late stages of amylotrophic lateral sclerosis (ALS).
ALS (also known as Lou Gehrig's disease) is a progressive disease of the nervous system. ALS specifically and progressively damages motor neurons, and the resulting denervation of muscular tissue in turn mediates muscular degeneration (e.g., dystrophy and atrophy). More specifically, muscular degeneration results from neuronal death, which occurs primarily by apoptosis, and the resulting denervation of muscles that normally receive axons from the affected motor neurons. The progressive muscular degeneration results in deficits in somatomotor function and speech and eventually is manifest as respiratory failure. Hence, it is crucial to identify effective therapies to prevent motor death and muscular degeneration. To date, SCI- and ALS-induced respiratory abnormalities have been neither successfully managed or treated due to a lack of effective therapeutic agents.